Brugada syndrome

Overview

Brugada (brew-GAH-dah) syndrome is a rare but potentially life-threatening heart rhythm condition (arrhythmia) that is sometimes inherited.

People with Brugada syndrome have an increased risk of irregular heart rhythms beginning in the lower chambers of the heart (ventricles).

Treatment of Brugada syndrome includes preventive measures such as reducing fever and avoiding medications that might trigger the arrhythmia. Some people with Brugada syndrome need a medical device called an implantable cardioverter-defibrillator (ICD).

Symptoms

Brugada syndrome often doesn't cause any noticeable symptoms. Many people with Brugada syndrome don't know they have it.

Signs and symptoms that may be associated with Brugada syndrome include:

• Dizziness
• Fainting
• Gasping and labored breathing, particularly at night
• Irregular heartbeats or palpitations
• Extremely fast and chaotic heartbeat
• Seizures

A major sign of Brugada syndrome is an irregular result on an electrocardiogram (ECG), a test that measures the electrical activity of the heart.

When to see a doctor

Brugada syndrome signs and symptoms are similar to those of some other heart rhythm disorders. It's important that you see a health care provider to find out if Brugada syndrome or another heart rhythm disorder is causing symptoms.

If you faint and you think it may be because of a heart condition, seek emergency medical attention.

If your parent, sibling or child has been diagnosed with Brugada syndrome, you may want to ask your provider if you should have testing to see if you're at risk of Brugada syndrome.

Causes

Brugada syndrome is a heart rhythm disorder. Each beat of the heart is triggered by an electrical signal made by special cells in the right upper chamber of the heart. Tiny pores, called channels, on each of these cells direct this electrical activity, which makes the heart beat.

In Brugada syndrome, a change in these channels causes the heart to beat too fast, creating a dangerous heart rhythm (ventricular fibrillation).

As a result, the heart doesn't pump enough blood to the rest of the body. An irregular rhythm that lasts for a short time can cause fainting. Sudden cardiac death can occur if the irregular heartbeat doesn't stop.

Brugada syndrome may be caused by:

• A structural problem in the heart, which may be hard to detect
• An imbalance in chemicals that help send electrical signals through the body (electrolytes)
• Use of certain prescription medications or cocaine

Risk factors

Risk factors for Brugada syndrome include:

• Family history of Brugada syndrome. This condition is often passed down through families (inherited). Having family members with Brugada syndrome increases a person's risk of having it, too.
• Being male. Men are more frequently diagnosed with Brugada syndrome than are women.
• Race. Brugada syndrome occurs more frequently in Asians than in people of other races.
• Fever. A fever doesn't cause Brugada syndrome, but it can irritate the heart and trigger fainting or sudden cardiac arrest in people with Brugada syndrome, especially children.

Complications

Complications of Brugada syndrome require emergency medical care. Brugada syndrome complications include:

• Sudden cardiac arrest. If not treated immediately, this sudden loss of heart function, breathing and consciousness, which often occurs while sleeping, is deadly. With fast, appropriate medical care, survival is possible.
• Fainting. With Brugada syndrome, fainting requires emergency medical attention.

Prevention

If someone in your family has Brugada syndrome, genetic testing can be done to determine if you have, or are at risk of, the condition.

Diagnosis

Brugada syndrome usually is diagnosed in adults and, sometimes, in adolescents. It's rarely diagnosed in young children because the symptoms are often unnoticed.

To diagnose Brugada syndrome, a health care provider will perform a physical exam and listen to the heart with a stethoscope. Tests are done to check the heartbeat and diagnose or confirm Brugada syndrome.

• Electrocardiogram (ECG) with or without medication. An ECG is a quick, painless test that records the electrical signals in the heart. During an ECG, sensors (electrodes) are attached to the chest and sometimes the limbs. The test can help detect problems with the heart's rhythm and structure.

  If a heartbeat is regular during the test, the next step might be wearing a portable ECG for a full day and night. This type of test is called a 24-hour Holter monitor test.

  Some people have symptoms of Brugada syndrome but the results of their initial ECGs and 24-hour Holter tests are within the standard range. These people may have additional ECGs that include medications given through an IV that can trigger an irregular heartbeat.

• Echocardiogram. An echocardiogram uses sound waves to create images of the heart. This test cannot diagnose Brugada syndrome on its own, but it can help identify structural problems with the heart.

• Electrophysiological (EP) testing and mapping. This test, also called an EP study, may be done in some people with suspected Brugada syndrome.

  In this test, a health care provider threads thin, flexible tubes (catheters) tipped with electrodes through the blood vessels to different areas within the heart. Once in place, the electrodes can show the spread of electrical signals through the heart.

Treatment

Treatment for Brugada syndrome may include medication, catheter procedures or surgery to implant a device that controls the heartbeat. Brugada syndrome treatment depends on the risk of having a serious irregular heartbeat (arrhythmia).

Being at high risk involves having:

• A personal history of serious arrhythmias
• Fainting spells
• Survived sudden cardiac arrest

If you have Brugada syndrome but no symptoms, you may not need specific treatment because the risk of a serious irregular heartbeat is likely low. However, a health care provider might recommend taking steps to reduce the risk of irregular heartbeats.

• Treat a fever aggressively. Fever is a known trigger of irregular heartbeats in people with Brugada syndrome. Use fever-reducing medications at the first sign of a fever.

• Avoid drugs that may trigger an irregular heartbeat. Many drugs can increase the risk of an irregular heartbeat, including certain heart medications and antidepressants. Too much alcohol also can increase the risk.

  Tell your health care provider about the medications you take, including drugs and supplements bought without a prescription.

• Avoid playing competitive sports. This might apply to people at high risk of a serious irregular heart rhythm. Ask your health care provider if you should avoid such sports activities.

Medications

Some people with Brugada syndrome are prescribed medications, such as quinidine, to prevent a potentially dangerous heart rhythm. These drugs may be prescribed alone or with a medical device — called an implantable cardioverter-defibrillator (ICD) — that controls the heartbeat.

Surgery or other procedures

People with Brugada syndrome who've had cardiac arrest or a worrisome fainting episode may need surgery or a catheter procedure.

• Implantable cardioverter-defibrillator (ICD). This small, battery-operated device is placed in the chest to continuously monitor the heart rhythm. It delivers electrical shocks when needed to control irregular heartbeats. ICD placement usually requires an overnight hospital stay.

  ICDs may deliver shocks when they're not needed, so it's important to discuss the benefits and risks of these devices with a health care provider.

• Catheter ablation. If an ICD doesn't effectively and safely control Brugada syndrome symptoms, a procedure called radiofrequency catheter ablation may be an option. A long, flexible tube (catheter) is inserted through a blood vessel and threaded to the heart. The catheter delivers high energy that scars or destroys the heart tissue responsible for the irregular heart rhythm.

If you have Brugada syndrome, you need regular health checkups to make sure the heart rhythm disorder is properly managed and controlled. Regular checkups can help your provider detect complications early and determine if a treatment change is needed.

Coping and support

Finding out you have Brugada syndrome may be challenging. You may worry about whether your treatment will work or whether other family members could be at risk.

Turning to friends and family for support is essential. If you find you need more help, talk to your health care provider about joining a support group. Connecting with others in a support group may help ease stress related to Brugada syndrome.

Preparing for your appointment

It's likely to take several appointments with your health care provider to confirm a diagnosis of Brugada syndrome and its severity. Your provider may give you instructions on how to prepare before each visit.

Here's some information to help you get ready for your first appointment.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance. For example, if you're having a test to look at your heart's electrical activity (electrophysiology test), ask your doctor how long you need to fast before your test.
• Write down your symptoms, including any that seem unrelated to Brugada syndrome, and when they began.
• Write down key personal information, especially any family history of sudden death, cardiac arrest or heart conditions, and any personal history of fainting or heart arrhythmias.
• Make a list of all medications, vitamins or supplements you take, including doses.
• Take a family member or friend along, if possible. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions for your health care provider.

For Brugada syndrome, some basic questions to ask include:

• What's the most likely cause of my symptoms?
• What tests do I need?
• What types of treatments are available for Brugada syndrome? Which do you recommend for me?
• What's an appropriate level of physical activity?
• How often do I need follow-up visits to monitor my condition?
• I have other health conditions. How can I best manage these conditions together?
• Are there brochures or other printed material I can take home with me? What websites do you recommend?
• Should my family be screened?

Don't hesitate to ask any other questions you have.

What to expect from your doctor

Your doctor is likely to ask you a number of questions, such as:

• Do you always have symptoms or do they come and go?
• How often do you have signs, such as fainting?